A Rare Case of Intraosseous Papillary Hemangioma of the Head and Neck

doi:10.21203/rs.3.rs-2602984/v1

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Case Report

A Rare Case of Intraosseous Papillary Hemangioma of the Head and Neck

https://doi.org/10.21203/rs.3.rs-2602984/v1

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Journal Publication

published 12 Jun, 2023

Read the published version in International Journal of Surgical Pathology →

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Objectives

Papillary hemangioma (PH) is a novel variant of intravascular capillary hemangioma. It is more common in adults and has a male predominance. Most tumors reported so far are solitary and cutaneous. Here we present a rare case of an intraosseous PH involving the frontal bone.

Methods

Brain imaging in a 69-year-old male with a slowly enlarging swelling on the right frontal area following an accidental fall demonstrated a 4.5 cm x 1.7 cm x 4.2 cm mass originating from the right frontal bone, with a tiny defect on the orbital roof. A malignant process was favored, and the mass was removed.

Results

Histopathology revealed a vascular lesion showing intraosseous distribution with foci of extension into the fibrous connective tissue. There were areas of plump endothelial cells with intracytoplasmic hyaline globules arranged in papillary configuration. The lesional cells were immunoreactive with CD34 and vimentin. AE1/AE3, EMA, PR, D2-40, inhibin and S100 stains were negative. Ki-67 was low.

Conclusion

This is the first intraosseous and second non-cutaneous PH. Clinically it differs from other cases by the presence of trauma as a preceding event. Since its prognosis is unknown such patients should be monitored for recurrence or malignant transformation.

Pathology

papillary hemangioma

intraosseous papillary hemangioma

non-cutaneous papillary hemangioma

glomeruloid hemangioma

capillary hemangioma

Papillary hemangioma (PH) is a novel variant of the intravascular capillary hemangioma that was first described in 2007. It has a male predominance. The median age of presentation is 55 years. Most of the tumors reported so far are solitary, cutaneous and located in the head and neck region. Here we present a rare case of an intraosseous PH involving the frontal bone.

A 69-year-old male presented with a swelling on the right forehead following an accidental fall. His past medical history was significant for type 2 diabetes mellitus, gout, hyperlipidemia, hypertension, and Wegener’s vasculitis (status post chemotherapy, hemodialysis, and plasmapheresis) under remission. As per the patient, the swelling was slowly enlarging in size. CT head demonstrated a 4.5 cm x 1.7 cm x 4.2 cm permeative mass originating from the right frontal bone (Fig. 1A), extending up to the superomedial aspect of the orbital rim and left frontal sinus and involving the superior and lateral cortex of the left frontal sinus. In addition, there was a tiny defect within the roof of the right orbit. Hence a malignant process such as osteosarcoma with microinvasion to extra coronal right orbit was favored by the radiologist. He underwent a right frontal craniectomy and resection of the skull mass with titanium mesh reconstruction and complex closure. Intraoperative pathology consultation favored meningeal neoplasm. Microscopic examination of the permanent sections revealed a vascular lesion showing intraosseous distribution with foci of extension into the fibrous connective tissue (Fig. 1B). There were areas of plump endothelial cells arranged in papillary configuration (Figure C). Intracytoplasmic hyaline globules and foci of extramedullary hematopoiesis were identified (Figure D). There was mild cytologic atypia, without atypical mitosis. The lesional cells were immunoreactive with CD34 and vimentin. Staining for AE1/AE3, EMA, PR, podoplanin, inhibin and S100 were negative. Ki-67 proliferation index was extremely low (< 1%). Given these findings a diagnosis of papillary hemangioma was established. The lesion was also present at the inked margin.

Hemangiomas are benign neoplasms of the blood vessels and are classified as capillary and cavernous. Capillary hemangiomas are further classified based on their structure into retiform, lobular, glomeruloid, fascicular, and papillary, pseudopapillary retiform. Its occurrence in the head and neck region is rare.¹ To our knowledge, there are only a total of 17 cases of PH reported so far.² It is one of the hemangiomas that is more common in adults than children³ and is more common in males than females (7:4). It presents as a slow growing, bluish skin papule. Most lesions are solitary, with the exception of one reported case that presented with multiple lesions over the chin.⁴ Though most lesions are cutaneous and found in the head and neck region, there has been one reported case of PH arising from the spinal cord.⁵ Our case of PH is the first intraosseous and second non-cutaneous lesion to be reported.

A diagnosis of papillary hemangioma is made by histopathological examination which is characterized by vascular endothelial proliferations in a branching papillary pattern. The endothelial cells are plump with intracytoplasmic hyaline globules. Immunohistochemical staining shows strong and diffuse expression of CD31 and CD34 by the endothelial cells and are negative for podoplanin. The intracytoplasmic hyaline globules are α1-AT positive. These lesions are always smooth muscle actin (SMA) positive, demonstrating the pericytes that line the intact basement membrane.¹ The main stay of treatment is excision, and the prognosis is excellent with very low recurrence rates and no evidence of malignant transformation.^1,2 Since prognosis of intraosseous PHs are unknown, our patient requires close clinical follow up to monitor for recurrences or malignant transformation.

The most common differential diagnosis is glomeruloid hemangioma.⁶ It can be differentiated clinically by the presence of multiple lesions and its association with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome.^6,7 Histologically, the architectural pattern of lesional cells resemble the renal glomerulus and can be best appreciated with type IV collagen staining. A few cases of glomeruloid hemangiomas were later reclassified as PH.^1,6

Our case of PH shares all the demographic, microscopic and immunohistochemical features of the cases that have been reported in the literature so far. However, a few differences have been noted. Our patient had a history of trauma to the region as a pre inciting event which has not been reported elsewhere. Also, as the lesion was non-cutaneous, our patient did not have a bluish papule which is classic of a vascular lesion. Since the clinical presentation, lesional site and radiographic evidence in our case was unique, a meningeal neoplasm was favored over a vascular lesion.

Papillary intraosseous hemangioma is a rare entity with very few reported cases. Ours is the second non-cutaneous and first intra-osseous lesion to be reported. In our case, the possibility of this lesion arising from bone could be related to his local trauma. Further studies regarding its histogenesis need to be explored. Prognosis of intraosseous PH is not known, and our patient is on close clinical follow up.

Conflict of interest statement:

The authors declare that they have no conflict of interest.

Funding statement:

This study was not supported by any funding.

Consent

Consent was waived based on institution regulations.

Suurmeijer AJ, Fletcher CD. Papillary haemangioma. A distinctive cutaneous haemangioma of the head and neck area containing eosinophilic hyaline globules. Histopathology. Nov 2007;51(5):638–48. doi:10.1111/j.1365-2559.2007.02847.x
Maloney N, Miller P, Linos K. Papillary Hemangioma: An Under-Recognized Entity Not to Be Confused With Glomeruloid Hemangioma. Am J Dermatopathol. Mar 2020;42(3):211–214. doi:10.1097/DAD.0000000000001546
Bancalari B, Colmenero I, Noguera-Morel L, Torrelo A, Enríquez de Salamanca J, Hernández-Martín Á. Papillary hemangioma in a child and sonographic characterization. Pediatr Dermatol. Jan 2020;37(1):233–234. doi:10.1111/pde.14048
Rammeh S, Fazaa B, Ajouli W, Labbene I, Kharfi M, Zermani R. Papillary haemangioma: a case report of multiple facial location. Pathologica. Jun 2014;106(2):67–9.
Ono Y, Goldsmith JD, Hsu N, Bryke C, Varma H. Papillary haemangioma in the spinal canal of a patient with a germline 12p microdeletion. Histopathology. 07 2019;75(1):151–153. doi:10.1111/his.13861
Suurmeijer AJ. Papillary hemangiomas and glomeruloid hemangiomas are distinct clinicopathological entities. Int J Surg Pathol. Feb 2010;18(1):48–54. doi:10.1177/1066896908323504
Chan JK, Fletcher CD, Hicklin GA, Rosai J. Glomeruloid hemangioma. A distinctive cutaneous lesion of multicentric Castleman's disease associated with POEMS syndrome. Am J Surg Pathol. Nov 1990;14(11):1036–46. doi:10.1097/00000478-199011000-00005

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Journal Publication

published 12 Jun, 2023

Read the published version in International Journal of Surgical Pathology →

Version 1

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You are reading this latest preprint version

A Rare Case of Intraosseous Papillary Hemangioma of the Head and Neck

Status:

Journal Publication

Version 1

Abstract

Objectives

Methods

Results

Conclusion

Figures

Introduction

Case Report

Discussion

Conclusion

Declarations

References

Additional Declarations

Status:

Journal Publication

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